We report a new case of Hyper-IgD syndrome. It's a rare disease, characteri
zed by recurrent episodes of fever, articular manifestations, abdominal pai
ns, vomiting, diarrhea, lynfoadenopathy, skin lesions, and headache. Polycl
onal increase of serum IgD is constantly present. The prognosis is benign.
The differential diagnosis between others diseases with periodic fever in c
hildren is discussed.